DOI: https://doi.org/10.36347/sjmcr.2025.v13i02.006

Pages: 240-245

Desmoid tumor is a benign fibrous neoplasia arising from the musculoaponeurotic structures of the muscles. They are characterized by their infiltrative and locally aggressive behavior with tendency for recurrence. Orbital location is extremely rare, only a few separate cases have been reported in the literature and are mainly pediatric. In this article, we report the case of an adult patient affected with a locally aggressive desmoid tumor involving the orbit that was unsuccessfully treated with hormonal therapy then by chemotherapy which induced a hematological toxicity before undergoing the ultimate surgical removal followed by adjuvant radiotherapy. The aim of the article is to describe this unusual location for desmoid tumors and outline the challenges in the decision-making process and the therapeutic alternatives. The management of desmoid tumors is mainly based on the function preservation, but in case of symptomatic patients or aggressive course of the disease, the surgical approach must be considered.